Pediatric tropical giant spleen syndrome
Introduction
Introduction to pediatric tropical spleen syndrome Tropical spleen syndrome (tropicalsplenomegalysyndrome), also known as tropical splenomegaly or idiopathic tropical splenomegaly, mainly characterized by chronic spleen, accompanied by hepatomegaly, white blood cells and thrombocytopenia, serum IgM and malaria antibodies are significantly elevated . basic knowledge The proportion of illness: the incidence rate is about 0.003%-0.005% Susceptible people: children Mode of infection: non-infectious Complications: hypersplenism
Cause
Causes of pediatric tropical spleen syndrome
(1) Causes of the disease
The onset of the disease is an immunoglobulin metabolic disorder caused by malaria infection, and the occurrence of this disease has genetic factors.
(two) pathogenesis
Tropical splenomegaly represents a host's abnormal response to parasites, especially malaria parasites. It is basically a disease of immunoglobulin metabolism disorder. Under long-term antimalarial treatment, the spleen shrinks and IgM declines. Related to malaria infection.
The lower serum complement C3, the presence of serum anti-complement activity and the presence of cold suspected globulin suggest that the disease is an immune complex disease, which can be used in the hepatic sinus Kupffer cells and macrophages by immunofluorescence. IgM and IgG and C3 are found on cells, and there are excessive immune complexes in the high malaria area due to repeated and persistent antigenemia. It takes many years for macrophages to clear these complexes, thus causing spleen and liver. Reticuloendothelial cell hyperplasia leads to enlargement of organs. Fakunle et al. (1976) considered that the regulation of immunoglobulins, especially IgM, by T lymphocytes is the main cause of this disease. Fakunle et al. (1978) in patients with this disease. In the middle, it was found that the proportion of blood T lymphocytes decreased, while the number of B lymphocytes increased, and the proportion of T cells in the spleen puncture fluid increased. Since the occurrence of this disease has racial and family attributes, there may be genetic factors regulating immunoglobulin regulation. .
Prevention
Prevention of pediatric tropical spleen syndrome
Prevention of malaria infection is a fundamental measure.
Complication
Complications of pediatric tropical spleen syndrome Complications, hypersplenism
The disease may have hypersplenism, hemolysis and portal hypertension.
Symptom
Symptoms of pediatric tropical spleen syndrome common symptoms portal hypertension low fever gastrointestinal bleeding low heat fatigue
The age of onset of this disease is from 8 to 65 years old. It rarely occurs before the age of 8 years. The clinical manifestations are low fever, anemia (mostly hemoglobin is 60-100g/L), and the liver and spleen are enlarged. The latter is especially obvious, and the bone marrow is obvious. Hyperplasia, anemia is mainly due to increased storage of blood cells in the spleen, hypersplenism, increased blood volume, hemodilution and repeated gastrointestinal bleeding, red blood cell life is slightly reduced, but can be dramatically reduced during pregnancy, some cases can be seen To hemolysis and portal hypertension.
Examine
Examination of pediatric tropical splenomegaly syndrome
1. Blood examination shows anemia in peripheral blood, and hemoglobin is significantly decreased, mostly in 60-100g/L, platelets can be reduced, and hemolysis can occur.
2. IgM is elevated in immune function test, complement C3 is low, serum anti-complement activity and condensation globulin are present, T lymphocyte ratio is decreased, and B lymphocytes are increased.
3. Pathological examination Fluorescence method can find IgM, IgG and C3 on Kupffer cells and macrophages of hepatic sinus, and the proportion of T cells in spleen puncture is increased.
4. Bone marrow examination, bone marrow, obvious hyperplasia
Should do abdominal B ultrasound and other checks.
Diagnosis
Diagnosis and diagnosis of pediatric tropical spleen syndrome
The diagnostic value of this symptom is that the serum IgM is significantly elevated and there is a high titer of malaria antibodies. Liver biopsy reveals lymphocytic infiltration in the liver sinus, spleen gradually shrinks after long-term antimalarial treatment, serum IgM declines and liver histology changes Better.
It is differentiated from portal hypertension and splenomegaly caused by cirrhosis.
